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Paroxysmal Hemicrania P1: KWW/KKL P2: KWW/HCN QC: KWW/FLX T1: KWW GRBT050-97 Olesen- 2057G GRBT050-Olesen-v6.cls August 17, 2005 1:30 ••Chapter 97 ◗ Paroxysmal Hemicrania Christopher J. Boes, Maurice Vincent, and David Russell PAROXYSMAL HEMICRANIA Sex Distribution PH was originally considered to be a female disease. Male International Headache Society (IHS) code and diag- cases, however, clearly occur (46,48). In initial series there nosis: was a female:male ratio of 7:1, but a 1989 review re- 3.2 Paroxysmal hemicrania ported a female:male ratio of 2.36:1 (1). Comprehensive 3.2.1 Episodic paroxysmal hemicrania and methodologically well-designed epidemiologic stud- 3.2.2 Chronic paroxysmal hemicrania ies are required to establish the actual extent of the female World Health Organization (WHO) code and diagnosis: preponderance in PH. G44.03 Chronic paroxysmal hemicrania Short description: Paroxysmal hemicrania (PH) suffer- ers have attacks with similar characteristics of pain Age of Onset and associated symptoms and signs to those of cluster PH may begin at any time, but its onset usually occurs headache (CH), but they are shorter lasting, are more during adulthood at the mean age of 34 years. The youngest frequent, occur more commonly in females, and re- age at onset was 1 year and the oldest 81 years (1,12). In spond absolutely to indomethacin. In episodic paroxys- the reviewed material from 1989, the mean age at diagnosis mal hemicrania (EPH), attacks occur in periods lasting was 47 and the mean illness duration 13 years. As far as 7 days to 1 year separated by pain-free periods lasting the subtypes are concerned, EPH seems to begin earlier 1 month or longer. In chronic paroxysmal hemicrania (mean 27 years) than CPH (mean 37 years) (1). (CPH), attacks occur for more than 1 year without re- mission or with remissions lasting less than 1 month (21). GENETICS Other terms: Sjaastad syndrome There is no evidence of a genetic etiology in PH. Parents EPIDEMIOLOGY or siblings do not have an increased incidence of CH or migraine compared with the general population (1). PH is a relatively rare disorder. Data on PH epidemiology are scarce, and many cases are probably still overlooked. Following the original description (58), new patients have ANATOMY, PATHOLOGY, been identified in several countries (1,25,57) and isolated AND PATHOPHYSIOLOGY PH cases are no longer reported. The incidence and preva- lence of PH are not known, but the relative frequency com- The pathogenesis of PH remains unknown. The unilateral- pared to CH is reported to be approximately 1 to 3% (1). ity and pain intensity, as well as the autonomic symptoms Prevalence estimates in CH vary from 56 per 100,000 to 381 and signs, suggest that some mechanisms may be shared per 100,000 (51,61,69). It has been reported that the preva- with the other trigeminal autonomic cephalalgias (TACs) lence of CH in the United States, based on one large study, listed in group 3 of the 2004 IHS headache classification. was 401 per 100,000 (51). However, this was a U.S. study The absolute indomethacin effect, however, indicates that of CH incidence, not prevalence, and converting incidence PH has a distinct pathophysiology. In most of the cases no to prevalence estimates is naturally combined with un- underlying pathology is detected by any supplementary in- certainties because survival time needs to be conjectured vestigation. Several secondary cases, however, have been (61,68). described (70) (Table 97-1). 815 P1: KWW/KKL P2: KWW/HCN QC: KWW/FLX T1: KWW GRBT050-97 Olesen- 2057G GRBT050-Olesen-v6.cls August 17, 2005 1:30 816 Tension-Type Headaches, Cluster Headaches, and Other Primary Headaches ◗ TABLE 97-1 Secondary Paroxysmal Hemicrania ular blood flow (23). The pulse synchronous changes in IOP are reflected by the corneal indentation pulse (CIP) ■ Vascular amplitudes, which may be measured in micrometers by ■ Circle of Willis aneurysm dynamic tonometry (22). The corresponding changes in ■ Occipital, middle cerebral artery infarctions intraocular volume in cubic milliliters can be estimated ■ Parietal arteriovenous malformation ■ Tumor using conversion tables. In PH there is a significant attack- ■ Malignant frontal tumor related increase in CIP amplitudes, ocular blood flow, and ■ Cavernous sinus meningioma IOP that is bilateral but more pronounced on the symp- ■ Petrous ridge meningioma tomatic side (55). The attack-related increase in IOP can- ■ Gangliocytoma of the sella turcica not be solely explained by changes in aqueous humor dy- ■ Pituitary adenoma namics because the increased volume exceeds turnover ■ Parotid epidermoid metastases of aqueous humor and the IOP changes occur so rapidly ■ Pancoast tumor (<30 seconds). Therefore, these findings are probably the ■ Miscellaneous result of an acute vasodilation that increases intraocular ■ Collagen vascular disease volume due to a neurogenic impulse and vasoactive neu- ■ Intracranial hypertension ropeptide release (55). ■ Maxillary cyst ■ Ophthalmic herpes zoster Since changes in IOP and CIP may indicate local abnor- ■ Essential thrombocythemia mal hemodynamics, spontaneous PH attacks were studied ■ Posttraumatic with transcranial Doppler (54). During attacks, there was hyperventilation, reduction of the end-tidal PCO2, and de- From refs. 5, 33, 70. crease in blood flow velocity in all insonated arteries, on both symptomatic and nonsymptomatic sides. In the ante- rior cerebral artery, the reduction was significantly smaller Although the pain in PH is strictly unilateral, autonomic on the symptomatic side. There was no major difference involvement and ocular signs may be bilateral, albeit more between patients and controls considering reduction of pronounced on the symptomatic side. The existence of bi- flow during hypocapnia, indicating that vascular reactivity lateral ocular signs seems to exclude the possibility of a in PH is normal. single lesion of the peripheral nervous system being the Corneal temperature has been found to be increased on cause of PH. A central lesion involving midline structures the symptomatic side during attacks, a finding that may seems therefore more probable (e.g., hypothalamus, cav- also be due to increased ocular blood flow following va- ernous sinus) (40,56). Functional neuroimaging studies sodilation (13). have shown specific activation of hypothalamic areas in The abruptness with which the accompanying au- some TACs, including CH and SUNCT (short-lasting uni- tonomic signs occur in mechanically precipitated at- lateral neuralgiform headache attacks with conjunctival tacks suggests that they may be mediated by neurogenic injection and tearing) syndrome (35,36,67). Similar neu- impulses. This would involve neuropeptide-containing roimaging findings in PH are not available. perivascular fibers of the trigeminovascular system with The pain in PH cannot be explained by a disturbance its connections to the cavernous sinus plexus and brain- in the autonomic nervous system alone. This is based on stem. The release of vasoactive peptides from sensory the observation that autonomic signs may precede the de- fibers, which run in close relationship to other autonomic velopment of pain in precipitated attacks and that the fibers, may also lead to miosis, increased IOP, and other pharmacologic suppression of autonomic signs does not autonomic disturbances observed in PH (14,37). Calci- influence the pain pattern during attacks (66). Besides, tonin gene–related peptide (CGRP), a peptide released the clinical picture does not fit with any classic sympa- from trigeminal fibers, and vasoactive intestinal polypep- thetic or parasympathetic syndrome. The pathophysio- tide (VIP), a parasympathetic peptide, have been found to logic mechanisms responsible for the pain in PH remain be abnormally high during a PH attack (18). Values re- unknown. It has been suggested that the ipsilateral distri- turned to basal levels following indomethacin treatment. bution of trigeminal fibers in the trigeminovascular system This is similar to the peptides’ profile found in CH (17), may explain the unilaterality of the pain, and evidence of suggesting that this disorder and PH may share patho- trigeminal–parasympathetic activation during PH attacks physiologic traits to a certain extent. It is possible that has emerged (18). the clinical picture in PH results from the interaction be- The ocular findings in PH patients have been studied in tween neurotransmitters and neuromodulators released detail with dynamic tonometry (22,23). Pulse synchronous from sympathetic, parasympathetic, and sensory fibers changes in intraocular pressure (IOP) may be recorded. at the frontal area and local autonomic and vascular They depend on the volume of the pulsatile part of oc- mediators. P1: KWW/KKL P2: KWW/HCN QC: KWW/FLX T1: KWW GRBT050-97 Olesen- 2057G GRBT050-Olesen-v6.cls August 17, 2005 1:30 Paroxysmal Hemicrania 817 The ocular vascular findings (increased IOP, conjuncti- The mechanism behind precipitation of attacks may val injection) may also be explained by autonomic changes. theoretically be due to reflex mechanisms involving con- The situation seems to be more intricate since experiments nections between the trigeminovascular system and brain- show that the IOP increase is inhibited by an α-blocking stem. However, local pathologic findings in the neck struc- agent (thymoxamine) as well as satellite ganglion blockade tures have not been found. (66). This observation, together with increased sweating In conclusion, the pathologic mechanisms behind the and decreased salivation on the symptomatic
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